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Unexpected and Deadly: Three Cases of Bone Marrow Necrosis.

Bone marrow necrosis (BMN) is a rare pathological diagnosis, with an incidence of 0.3% to 2%. It is most often associated with hematological malignancies and less commonly due to solid tumors, infections, medications, sickle cell disease, chemotherapy, radiotherapy, or idiopathic causes. We reviewed bone marrow biopsies performed in our institution from 2009 to 2019 and found three cases of BMN. Two cases were secondary to neoplastic causes, while the third one was possibly from alcohol abuse.

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… Reticulin and trichrome stains both showed moderate fibrosis. Iron stain showed a normal amount of stainable iron (Figure 1 ). … Abundant stainable iron and mild reticulin fibrosis were found. No evidence of infection or neoplasia. Clinically, she had no evident source of bleeding. She was discharged with stable hematogical parameters and advised close follow-up and alcohol cessation. …

Selinexor plus ruxolitinib in JAK inhibitor-naïve patients with myelofibrosis: a multicenter, open-label, phase 1 study.

AbstractThe phase 1 portion of SENTRY/XPORT-MF-034 (NCT04562389) evaluated the exportin 1 inhibitor selinexor (40 mg/60 mg once weekly) plus ruxolitinib in JAK inhibitor-naïve patients with myelofibrosis (n = 24). Primary end points were maximum tolerated selinexor dose, recommended clinical trial dose, and safety. No dose-limiting toxicities were reported. Common adverse events were nausea, fatigue, anemia, thrombocytopenia, constipation, vomiting, and headache. Nausea was transient, mainly grade 1, and managed by prophylactic antiemetics. Four deaths occurred, all unrelated to study treatmen

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… Slide pairs were stained centrally for reticulin, CD61, and CD71. … Based on the central pathologist review of reticulin-stained slides, MF grade was stable at week 24 in most patients, and only 2 worsened (1 from MF-1 to MF-2, 1 from MF-0 to MF-3). …

Anemia in elderly patients admitted in the geriatric and medicine wards of S. S. hospital: A cross-sectional study.

INTRODUCTION: Regardless of its cause, anemia is a very prevalent condition linked to higher morbidity and a lower quality of life. The difficulties that result from anemia make it more severe, especially in the elderly population. Assessing the clinicopathological range of geriatric anemia, which frequently goes undiagnosed, is the aim of our study. MATERIAL AND METHOD: The present study is a prospective hospitalbased cross-sectional study conducted between July 2019 and January 2022. Total of 102 patients aged 60 years and above were included in the study. Detailed demographic, clinical, an

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… Reticulin stain and hematoxylin and eosin were used to stain the biopsy sections. Statistical analysis Statistical analysis was performed by Statistical Package for the Social Sciences version 23.0. Categorical variables were expressed as frequencies. … (c) Trephine Biopsy showing myelofibrosis (Reticulin stain, magnification 10x). (d) Bone Marrow Aspiration smear showing Megaloblast.(Leishman stain, original magnification 100x). …

Multi-focal Lytic Lesions in a Patient with Myelofibrosis: A Case Report.

Myelofibrosis is a rare disorder that is classified as one of the myeloproliferative disorders. This particular disorder results in the abnormal proliferation of hematopoietic stem cells in the bone marrow. In some cases, such as ours, pathologic fractures can occur due to skeletal manifestations. We report on a rare finding of rapidly progressive lytic lesions in multiple regions throughout the body. This presentation of myelofibrosis behaving in a metastatic-like fashion has not been previously described.

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… Immunohistochemistry stain for CD61 highlights megakaryocytes (Figure 2B ) and reticulin stain demonstrates extensive marrow fibrosis (Figure 2C ). Thus, the final diagnosis of myelofibrosis was concluded. Figure 2 Left femur histologic sections. … Histologic section (A), immunohistochemistry stain for CD61 (B), and reticulin stain (C) of the left proximal femur. The patient underwent left femur intramedullary nail fixation followed by palliative radiation therapy, 25 Gy in 10 fractions. …

The clinical picture of Castleman disease: a systematic review and meta-analysis.

AbstractCastleman disease (CD) encompasses a spectrum of rare disorders, including unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD). We performed a systematic review of publications reporting ≥5 cases of CD between 1995 and 2021, following preferred reporting items for systematic reviews and meta-analyses guidelines, to describe and compare subtypes. We extracted data on clinical symptoms and laboratory parameters as stated in international consensus diagnostic criteria for iMCD and estimated the frequency of each criterion using meta-a

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… NOS, not otherwise specified; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; TAFRO, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly; TB, tuberculosis. Histopathological features of CD have been well described. … serology, HHV8 qPCR (peripheral blood), EBER (lymph node), and LANA-1 (lymph node) Cytokine profile | Interleukin-6 and vascular endothelial growth factor Imaging | CT-PET or CT neck, chest, abdomen, and pelvis Bone marrow evaluation | Monoclonal gammopathy of undetermined significance, myeloma, and reticulin …

Emerging treatments in Castleman disease - a critical appraisal of siltuximab.

Castleman disease (CD) is a rare, heterogeneous lymphoproliferative disorder for which no standard of care currently exists. Evidence that the pathophysiology of CD is fueled by excessive interleukin-6 (IL-6) has led to considerable interest in therapeutic targeting of this cytokine. Siltuximab, a chimeric monoclonal antibody to IL-6, has thus emerged as a promising treatment option in a disease lacking efficacious therapy. Here, we review the findings of recent studies evaluating single-agent siltuximab treatment in CD, including the first-ever randomized clinical trial in this disease. Altho

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… 6 Tsukamoto Y Hanada N Nomura Y Rapidly progressive renal failure associated with angiofollicular lymph node hyperplasia Am J Nephrol 1991 11 430 436 1809043 10.1159/000168351 7 Inoue M Ankou M Hua J Iwaki Y Hagihara M Ota Y Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin … fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: a case report J Clin Exp Hematop 2013 53 95 99 23801140 10.3960/jslrt.53.95 8 Takai K Nikkuni K Momoi A Nagai K Igarashi N Saeki T Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca …

Janus kinase-1 and Janus kinase-2 inhibitors for treating myelofibrosis.

BACKGROUND: Myelofibrosis is a bone marrow disorder characterized by excessive production of reticulin and collagen fiber deposition caused by hematological and non-hematological disorders. The prognosis of myelofibrosis is poor and treatment is mainly palliative. Janus kinase inhibitors are a novel strategy to treat people with myelofibrosis. OBJECTIVES: To determine the clinical benefits and harms of Janus kinase-1 and Janus kinase-2 inhibitors for treating myelofibrosis secondary to hematological or non-hematological conditions. SEARCH METHODS: We searched the Cochrane Central Register of…

Novel Molecular Therapies and Genetic Landscape in Selected Rare Diseases with Hematologic Manifestations: A Review of the Literature.

Rare diseases affect less than 1 in 2000 people and are characterized by a serious, chronic, and progressive course. Among the described diseases, a mutation in a single gene caused mastocytosis, thrombotic thrombocytopenic purpura, Gaucher disease, and paroxysmal nocturnal hemoglobinuria (KIT, ADAMTS13, GBA1, and PIG-A genes, respectively). In Castleman disease, improper ETS1, PTPN6, TGFBR2, DNMT3A, and PDGFRB genes cause the appearance of symptoms. In histiocytosis, several mutation variants are described: BRAF, MAP2K1, MAP3K1, ARAF, ERBB3, NRAS, KRAS, PICK1, PIK3R2, and PIK3CA. Genes like H

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… Thrombocytopenia, ascites/anasarca, myelofibrosis/fever, renal dysfunction/reticulin fibrosis, and organomegaly (TAFRO) constitute a distinct clinicopathological form of idiopathic HHV8/KSHV-negative Castleman disease with mixed histological features of hypervascular and plasmacytoid lymphocytes [ 20 … This iMCD patient also exhibited clinicopathological features corresponding to a specific subtype known as Castleman-Kojima disease (thrombocytopenia, anasarca, fever, reticulin fibrosis, and the clinical subtype of organomegaly [TAFRO]). …

T-Cell Lymphoma With Sarcoid-Like Reaction and Secondary Myelofibrosis: A Unique Case Report and Literature Review.

INTRODUCTION: T-cell lymphomas are rare, comprising 10%-15% of non-Hodgkin lymphomas (NHLs). Their etiology remains unclear, and pathophysiology varies widely among subtypes. Sarcoid-like reactions (SLRs) have been reported in approximately 7.3% of NHLs, including T-cell lymphomas, and can closely resemble sarcoidosis clinically/histologically. This overlap complicates diagnosis, particularly when secondary myelofibrosis-a rare and poorly understood complication-arises. Myelofibrosis has also been observed in sarcoidosis, further blurring the distinction between these entities. Treatment for T

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… Core biopsy demonstrated a hypercellular (“packed”) marrow with diffuse marked myelofibrosis (MF‐3 reticulin fibrosis), decreased trilineage hematopoiesis, noncaseating granulomata, and an atypical T‐cell infiltrate. … (C) Reticulin stain showing diffuse fibrosis. FIGURE 2 (A) Flow cytometry showing CD3 dim positive and TRBC1 negative. (B) T‐cell gene rearrangement (TCGR) showing probe set with prominent peaks. 3. …

Artificial Intelligence in Bone Marrow Histological Diagnostics: Potential Applications and Challenges.

The expanding digitalization of routine diagnostic histological slides holds a potential to apply artificial intelligence (AI) to pathology, including bone marrow (BM) histology. In this perspective, we describe potential tasks in diagnostics that can be supported, investigations that can be guided, and questions that can be answered by the future application of AI on whole-slide images of BM biopsies. These range from characterization of cell lineages and quantification of cells and stromal structures to disease prediction. First glimpses show an exciting potential to detect subtle phenotypic

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… This initial evaluation is generally performed on a hematoxylin and eosin (H&E)-stained glass slide, supported by additional histochemical stains (PAS, Giemsa, Perls; reticulin) for the assessment of specific cell features and lineages and stroma components. … In a recent study, Ryou et al. [ 36 ] used reticulin fiber-stained WSIs to develop algorithms to visualize and grade areas with increased fibers in WSIs of MPN and showed good correlation with morphological grading and MPN type. …

TAFRO syndrome: current perspectives.

Multicentric Castleman's disease (MCD), a distinct subtype of Castleman's disease, is a rare, nonneoplastic, lymphoproliferative disorder. Patients with MCD present with systemic symptoms and multiple lymphadenopathy. Lymph node biopsy is necessary for the diagnosis of various histological MCD patterns including hyaline vascular, plasma cell, and mixed types. Human herpesvirus 8 (HHV8) infection was identified as an important etiology of MCD among immunocompromised patients such as those positive for human immunodeficiency virus. Although HHV8-negative MCD was reported in immunocompetent patie

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… Reticulin fibrosis was assessed by bone marrow biopsy. 22 The first international consensus diagnostic criteria for iMCD The lack of established diagnostic criteria or disease-specific biomarkers for iMCD might hinder clinicians from reaching the correct diagnosis and starting treatment before the … 1827.2001.01266.x 18 Dossier A Meignin V Fieschi C Boutboul D Oksenhendler E Galicier L Human herpesvirus 8-related Castleman disease in the absence of HIV infection Clin Infect Dis 2013 56 6 833 842 23223599 10.1093/cid/cis1009 19 Takai K Nikkuni K Momoi A Nagai K Igarashi N Saeki T Thrombocytopenia with reticulin …

A phase 1 study of IO-202, an anti-LILRB4 antibody, in chronic myelomonocytic leukemia and acute myeloid leukemia.

IO-202 is a humanized immunoglobulin G1 monoclonal antibody with high affinity and specificity for leukocyte immunoglobulin-like receptor B4 (LILRB4; ILT3), which is predominantly expressed in monocytes and monocytic blasts. IO-202 induces antibody-dependent cellular cytotoxicity and antibody-dependent cellular phagocytosis in vitro and in patients with leukemia. Herein, we present the phase 1a dose escalation data of IO-202 as monotherapy and in combination with azacitidine (AZA) in patients with relapsed/refractory (R/R) acute myeloid leukemia (AML) and R/R chronic myelomonocytic leukemia (C

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… (8) MPN-SAF TSS >20, % (n) 38.1 (8) MPN-SAF TSS >15, % (n) 47.6 (10) >50% LILRB4-positive blasts and >4500 copies per cell, % (n/no. of patient with data available) 31.6 (6/19) High risk by CPSS-Mol (score ≥4), % (n/no. of patient with data available) 45 (9/20) Abnormal cytogenetics, % (n) 19.1 (4) Reticulin … MPN-SAF TSS >20, % (n) | 38.1 (8) MPN-SAF TSS >15, % (n) | 47.6 (10) >50% LILRB4-positive blasts and >4500 copies per cell, % (n/no. of patient with data available) | 31.6 (6/19) High risk by CPSS-Mol (score ≥4), % (n/no. of patient with data available) | 45 (9/20) Abnormal cytogenetics, % (n) | 19.1 (4) Reticulin …

Немногодействующий клеточный ассоциированный гематотоксичный с вторичным миэлофиброзом после Идекабтаген-Виклеукеля для множественной миеломы, полностью обработанный аутентичным гематопоэтическим сетком.

Panktopenia в сочетании с химериковой антигенной рецепторной клеткой T (CAR-T) называется иммунной гематопоиетической токсичностью (ICAHT), которая часто устойчива и противодействует поддержке. Мы сообщаем о случае 52-year-old Человек с множественной миеломой, который развил серьёзный ИКАХТ с вторичным миэлофиброзом (SMF) после введения идекабатагена-виклеукаля, который был полностью усовершенствован за счет аутологического гематопоиетического усиления стволовых клеток. В данном случае основное внимание уделяется СМФ как редким, но осторожным осложнениям CAR-T-терапии, что подчеркивает важност

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… A bone marrow biopsy performed on Day 25 after ide‐cel infusion revealed increased reticulin fibrosis consistent with Grade 2 myelofibrosis (Figure 1A–C ). …

Проблемы с диагностикой и оказанием неотложной медицинской помощи в случае быстро прогрессирующего заболевания лёгких, вторичного и многократного заболевания миеломой.

Одновременное представление множественной миеломы (ММ) и межститиальной болезни легких (МЛ) остается исключительно редким, при этом ограниченная литература описывает оптимальные стратегии управления для этой двойной патологии. Мы сообщаем о сложном случае 62-year-old Мужчина с недавно поставленным диагнозом MM с цитогенетикой промежуточного риска (del13q, набирающий 1q), который одновременно представил быстро прогрессирующий ILD, демонстрирующий обычную интерститальную пневмонию (МСАГВ). Пациентка 6-month Клинический курс был отмечен участием многих организаций, периодическими инфекциями, тром

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… On further evaluation, bone marrow biopsy confirmed the diagnosis with findings of diffuse plasma cell involvement with both mature and immature forms (plasmablasts) in sheets, grade 0 reticulin fibrosis, and cortico-cancellous bone architecture. …

Non-secretory Multiple Myeloma: A Report of a Rare Case.

Multiple myeloma (MM) is a rare blood cancer. Non-measurable MM (NMMM) is a category designation consisting of both non-secretory MM (NSMM) and oligo-secretory MM (OSMM), both of which have negative findings on serum protein electrophoresis and urine protein electrophoresis testing. NSMM and OSMM are infrequent subtypes. This report describes the case of a 67-year-old female patient without significant past medical history who presented with back pain, nausea, fatigue, and mild confusion. Labs were significant for severe hypercalcemia. Imaging demonstrated osteopenia and multiple lytic lesions

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… A bone marrow biopsy was obtained and demonstrated plasma cell myeloma (cellularity: average 70%; plasma cells: 75%) with background bone marrow demonstrating reduced trilineage hematopoiesis and moderate reticulin fibrosis but no increase in the number of blasts (<1%). …

Identification of Fatty Acid Metabolism Disorder-Related Gene Signature in Septic Cardiomyopathy.

Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations, and outcomes. In this retrospective international study, we analyzed 116 adult patients followed at 13 European ter

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… At bone marrow evaluation, most patients displayed erythroid and megakaryocytic hyperplasia (n = 27 and 18, respectively), 39% had dysplastic features of either erythroid or megakaryocytic lineage, and 24% had an increase of reticulin fibrosis (World Health Organization grade MF1). … The latter included 1 panel for the assessment of mutations in genes commonly mutated in myeloid neoplasms, performed in 20 patients with some features of bone marrow failure (hypoplasia, dyserythropoiesis, and reticulin fibrosis). …

Gaucher disease: the hematologist's perspective of a multisystemic disorder.

Gaucher disease (GD) exemplifies how a single genetic mutation can give rise to a complex multisystem disorder with profound hematological implications. Central to its pathophysiology is the lysosomal accumulation of glucosylceramide due to deficient glucocerebrosidase activity, together with abnormal folding and trafficking of the enzyme that induce endoplasmic reticulum stress and cellular dysfunction. These processes disrupt reticuloendothelial homeostasis and interfere with hematopoiesis. As a consequence, macrophage activation and chronic inflammation contribute to the cytopenias, splenom

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… Bone marrow biopsy revealed hypercellularity (90%) with diffuse infiltration by CD68-positive histiocytes and no evidence of reticulin fibrosis. … often increased Variable cellularity; dense reticulin and/or collagen fibrosis; atypical megakaryocytes (hypercromatic, bulbous or “cloud-like” nuclei); frequent dry-tap Paratrabecular or nodular infiltration by small B-cells; possible plasmacytoid differentiation Other features Bone pain; neuro signs …

Multiple Myeloma Plasmacytoid Infiltration of the Gallbladder Presenting as Acute Cholecystitis.

Secondary distal plasmacytoma that arise from multiple myeloma is a common hematological malignancy that manifests in later stages of the disease. These plasmacytomas are known to cause extensive systemic organ damage. When extramedullary plasmacytomas reach the gallbladder or biliary ducts, expansion can mimic acute acalculous cholecystitis or cholangiocarcinoma. We report a case of a 56-year-old female with a history of aggressive course IgA κ chain multiple myeloma who presented with right upper quadrant abdominal pain with nausea and vomiting for one week duration. Her laboratory panel rev

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… IgG Total | 219 mg/dL | (normal 694 – 1618 mg/dL) IgA Total | 10,862 mg/dL | (normal 81 – 463 mg/dL) IgM Total | 18 mg/dL | (normal 48 – 271 mg/dL) Immunofixation electrophoresis: paraproteins | Detected Immunofixation electrophoresis: interpretation | Data suggests monoclonal gammopathy: IgA type κ Reticulin …

An Unusual Case of Primary Plasma Cell Leukemia Presenting As Pericardial Effusion.

Plasma cell leukemia represents the rarest form of plasma cell dyscrasias arising from monoclonal proliferation of plasma cells in peripheral blood. Its aggressive natural history raises a significant therapeutic challenge. We present a case of a 58-year-old female who presented with a cough accompanied by exertional dyspnea. Further radiological investigations revealed a circumferential pericardial effusion. A pericardiocentesis was performed, resulting in the drainage of 650 cc of hemorrhagic pericardial fluid. Cytology revealed monoclonal plasma cells expressing CD38 and CD138 with cytoplas

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… Following these findings, a bone marrow biopsy confirmed a hypercellular marrow 95% with a plasma cell population of 33%, mild reticulin fibrosis, absent sustainable iron, and negative Congo-red stain for amyloid deposition (Figure 3 ). …

Idiopathic multicentric Castleman disease treated with siltuximab for 15 years: a case report.

UNLABELLED: Human herpes virus-8 (HHV8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder sustained by pro-inflammatory cytokines, including interleukin-6 (IL-6). According to the international evidence-based criteria developed by the Castleman Disease Collaborative Network (CDCN), siltuximab, which works by inhibiting IL-6, is the recommended choice for iMCD treatment. We report a case of a 63-year-old white male with iMCD who has been on maintenance therapy with siltuximab for 15 years - representing one of the longest treatment periods of any

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… The patient tested negative for antinuclear antibodies and no reticulin fibrosis was present. Evaluation of the patient found no evidence of monoclonal gammopathy. …